Hannah Clark was
rejecting her donor heart

Lancet: Girl Well After Transplanted Heart Removed

July 17, 2009
by Lynn Shapiro, Writer
In a medical feat, a British girl named Hannah Clark--who had a donor heart grafted onto her own after suffering heart failure as a baby--has made a full recovery after having the transplanted heart removed.

The operation to remove the donor heart took place 10 years after it was transplanted, and was possible because her own heart had recovered sufficiently to operate on its own.

Now, more than three years after this second operation, Hannah's remarkable story, from baby to present day (she is now 16 years old), is reported in an article published online and in an upcoming edition of The Lancet, written by leading heart surgeons Professor Sir Magdi Yacoub, Imperial College London, Heart Science Centre, Harefield Hospital, Middlesex, UK, and consultant Victor Tsang, Great Ormond Street Hospital, London, UK and colleagues.

Heart transplantation is a life-saving procedure in infants and small children with heart failure due to cardiomyopathy (a problem with the muscle of the heart). This condition occurs in 1.2-1.4 children per 100,000, and is 8 to 12 times more common in the first year of life than the subsequent years. The prognosis is very poor. However, it is theoretically possible for a patient's own heart to recover if he or she lives long enough for this to occur. Other options, such as mechanical hearts suitable for use in children, are currently being developed.

Hannah was brought to Harefield Hospital, UK (now part of the Royal Brompton & Harefield NHS Foundation Trust), in 1994. She had symptoms of severe heart failure secondary to cardiomyopathy. At age 2 years (in 1995) Hannah underwent a heart transplant, in which the donor heart was grafted onto, and took over much of the function of, her own heart. The new heart was inserted and functioned in parallel to Hannah's own heart.

The healthy, implanted heart assisted in pumping blood, which meant that Hannah's own heart could "rest" and gradually recover. However, as with all transplants of donor organs, the recipient's immune system must be suppressed using drugs to prevent rejection of the donated organ. Immunosuppression causes a very large increase in the incidence of malignant disease, particularly a type of cancer called Epstein-Barr-virus-associated post-transplant lymphoproliferative disorder (EBV PTLD). EBV is the virus which causes glandular fever in healthy individuals.

Some four and a half years after the transplant, both the donor heart and Hannah's own heart were functioning normally. However at this stage, removal of the donor heart--a major operation--was not deemed necessary. However, Hannah continued to have episodes of EBV PTLD, which by age 8 years had begun to spread and become extremely serious. Chemotherapy and multiple courses of drugs were used to get the EBV PTLD into complete remission. However, in January 2003, she developed symptoms suggesting that PTLD had recurred, and she went through similar drug cycles to treat it. This continued for two more years.

Then, in 2005, an echocardiogram showed that while her own heart was functioning normally, the function of the donor heart had become impaired. This was because doctors had needed to reduce Hannah's immunosuppressant drugs to help her fight PTLD, leading to symptoms of rejection of the donor heart. And since her PTLD was seemingly incurable, doctors decided to remove the donor heart so that immunosuppression could be stopped altogether. This was a medical "first".

Surgery to remove the donor heart took place at Great Ormond Street Hospital, London, UK, in February, 2006, just over a decade after the original operation. The surgery team was led by consultant Victor Tsang and Professor Sir Magdi Yacoub. Following surgery, the immunosuppressant drugs were removed altogether, and Hannah has since made a complete recovery from the EBV PTLD.

The authors say: "Her post-operative course was uncomplicated and the outcome was excellent. Three and a half years after surgery, the patient remains well, in complete remission from her PTLD, and has normal cardiac function."

Professor Yacoub adds: "Apart from the overriding human element in this report, Hannah's case has provided many lessons relevant to biology, transplantation, heart recovery and malignant disease. We all hope that this will stimulate further research and progress in this area."

Tsang adds: ""Hannah's case highlights that in cases of infant cardiomyopathy such as hers, it is possible for the patient's own heart to make a full recovery if it is given adequate support to do so. This is an important piece of knowledge as we are now gaining more experience with mechanical support for the failing heart in children."

Hannah Clark says: "Thanks to this operation, I've now got a normal life just like all of my friends. I've just done my GCSEs (examinations for a General Certificate of Secondary Education), and I've now got a Saturday job looking after animals, which I couldn't have done before. I'm really glad that I don't have to rely on life-saving drugs anymore."

For full Article, see: http://press.thelancet.com/cardiectfinal.pdf
For diagram (see: http://press.thelancet.com/hannahhearts.jpg

Source: Lancet Press Release